United Brachial Plexus Network, Inc.

Hi,

What is the ideal time frame for a child to have the ACR (what age range)?

Thanks in advance!

Jody - mom to Alyssa (9) LOBPI

The answer is young, and the younger the better. The ACR works by helping the ball of the humerus shift in the socket and changes the forces on the growing joint. The glenoid and humeral ball then have to grow out to accomodate the changed forces. The glenoid will grow more at the back than the front, levelling out the "socket" part of the joint. The humeral ball, which also is typically deformed and not round, also grows out into a more rounded shape. So there must be adequate growth left, that this normalization of the joint surfaces has time to occur. In addition, the longer the joint is subluxed and growing in malformed geometry, the longer all the other muscles and ligaments have to accomodate the situation, which will limit the gains obtainable from this surgery.

If the ACR is done too late, all you do is destabilize the joint but there isn't enough growth for it to restabilize with a normalized ball and socket. In addition, just shifting the ball in the socket may not be enough in an older child, where the entire musculature has already accomodated a malformed shoulder joint. This would limit recovery, even if the ball-and-socket joint does normalize in shape. Basically, if done too late, the ACR might introduce more problems than it would solve.

If I were looking at "pushing the envelope" on age for this procedure, I would definitely talk to doctors who have been doing it the longest and get real, concrete data on how their children, especially older children, benefit. I think the upper age limit depends on how much deformity there is, as well - a more deformed joint takes longer to "grow out" into the right formation and might require an excessive amount of tissue released in order to get it back "in", resulting in an unstable joint. I've seen 8 years given as one cutoff, but it depends on how much malformation there is that needs to correct itself.

Most important is the surgeon's experience with correcting subluxations and glenoid malformation with this procedure. Keeping tabs on exactly what was done and what were the results (juding by followup MRIs) has been critical for understanding the causes-and-effects from ACR. It is a delicate balance: there is a very real risk for loss of internal rotation, and the docs who have been doing it the longest have adjusted their approaches to reflect this. But if they don't release enough, the joint stays subluxed. Release too much capsule and you might introduce long term anterior instability, etc! It's a LOT to consider!

Then there is also the choice between arthroscopic and open procedures. They really are not the same procedure at all, because with an open capsule release in order to even access the capsule the anterior muscle tendons (pectoralis and possibly conjoined lat/teres major tendon) must be released. This adds additional risk for loss of internal rotation that can be minimized by an arthroscopic approach which only selectively releases what is absolutely necessary. The balance in an open procedure also becomes different; because of the additional tendon and muscle releases, the capsule might have to be released less (to preserve internal rotation) resulting in less normalization of the glenoid. If you release too much of the rotator interval tissue, there might be nothing left to hold the joint in place and the child could be left prone to anterior dislocation later in life.

This is one surgery where you want to look at statistics and what success the surgeon and others performing the surgery have had, in terms of post-op function (losses and gains) as well as solid tracking of joint geometry and improvement. This is a great case where by collaborating much more can be learned more quickly; with similiar tracking and surgical protocols, the different doctors performing this surgery arthroscopically, I know, have been able to take advantage of all the kids who have had it, and not just their own set of patients. This has greatly increased all their knowledge and the ability to predict results with more accuracy.

It is all about odds - the results are not what it immediately seen on the operating table, but in how the body responds over time to the surgery, and reforms the joint (or doesn't). You are basically betting that your child will respond in a similar way as other kids, that that result will be better than what she could have with other surgeries (such as osteotomy) and the surgeon should have very solid grounds for making these predictions.

Kate

Hi Kate,

Thanks for the response. Sounds like you know what you are talking about, and why I am asking the question. I can see what you mean by it not reforming itself, and there is no assurance that it can or will. What in your "opinion" do you think an older child with a malformed shoulder/joint should have done? Anything? Nothing? I just don't know if there is anything that can be done for a child her age with these issues.

Thanks again,

Jody

Would follow-up MRI's clearly show sublux shoulders, or would a child need other tests to determine this?

Jody, we are battling the same questions - what should be done to a subluxated shoulder that's malformed and additional secondary issues have begun (pain, etc.). In my search for answers I received a number of different opinions. (1) ACR should be done by age 4 for the long term effects of remolding and staying in position, they also said possibly until age 6 and that's probably based on what the MRI shows and (2) that when the child has excess weight, this is a great hinderance to the long term positive effects of any surgery so getting on top of this is paramount and (3) you can rarely have it both ways with a severe injury - you will end up losing internal rotation or external rotation, so choose which is more important to have. So if Maia can play her instruments now and it's her emotional sanctuary - a surgery might help her shoulder but she wouldn't be able to play her instruments. That's just horrible.

I am still searching for a soft tissue answer for Maia. Early on we were told that if we did this, this and that then she could avoid having a humeral osteotomy. So I became scared of osteotomies - they were something we had to work against. So no matter how many opinions we get to have humeral osteotomy, I just can't wrap my mind around it and although I do understand how rotating the deltoids when you rotate the bone can help the shoulder, too, I want to truly understand why some children have failed osteotomies. Is it about who did it, how they did it, something about the child's anatomy - what is it exactly?

And to add to that, one opinion is that humeral osteotomy shouldn't be done before age 13 (puberty).

And on a side note I was also told that swimming was paramount - 3X a week minimally.

The differing opinions have me so confused that all I can do for Maia right now is keep her elbow splinted at night and when something makes sense to me we'll move on it. She's going to have an MRI in two weeks and I'm looking forward to see if there's any more (negative) change to the position of her humeral head.

Taneesha,

Follow-up MRIs should clearly show the condition of the joint, IF taken using a standardized protocol so the joint is visualized properly and the proper comparisons can be made. Joshua is due for a 3-year followup MRI next June. His 1-year showed significant improvement, which is usually the case. The most dramatic improvement happens the first year and then it is gradual from there. If the 1-year post-op MRI doesn't show dramatic improvement, then the remodelling is unlikely to be very good.

Jody and Francine,

I wish there were good answers to this. The ACR is a very unique surgery, in that the effects on the body are being so closely watched and quantified. Other surgeries might have long-term "functional" outcomes tracked very closely, but none watch how the body adjusts to the surgery the way ACR does. If I was in your position, I would consider an osteotomy but ask very pointed questions about exactly where the bone would be cut and why, and what the surgeon thinks actually happens within the joint for kids whose internal rotation contractures return.

I wish there was more understanding about how the osteotomy interacts with the deformed glenohumeral joint, so that there were better answers to this question.

Kate

Yes, I wish that, too. Even after all these years since Maia's birth there is still so much that is unknown.

Does every child who has the ACR completely lose internal rotation at some point?

All kids lose some internal rotation, but I have never heard of a child *completely* losing internal rotation. That would mean they couldn't get their arm in from a "stop sign" position or in from out to the side. That would be severely debilitating and I've not heard of anything that extreme. Some kids have lost too much internal rotation - due to other releases and transfers being done at the same time, too much subscapularis released, or just the ACR "revealing" a fundamental weakness of the subscapularis (as we believe is the case with our child, since his subscap was minimally released). Some kids have needed a "re"rotational osteotomy to balance things out. This is one reason to be very cautious with this surgery and not do too much all at once. And then, of course, kids are stiff coming out of being immobilized in external rotation for a period of weeks, but that isn't the same thing.

Our son did lose some internal rotation, but as our son had this surgery at 13 months of age, it is difficult to tell what he would have been able to do at midline if he'd not had the surgery. Yes, his arm was positioned (stuck!) at his belly but that doesn't mean he would have had any strength in that position to be functional there. There is a difference between a contracted "range" where the arm is turned inward, and a functional range where the hand can be pressed against the body. A child may be stuck in an internal rotation posture but still be very weak and unable to manipulate anything in internal rotation.

But even if he lost internal rotation that he would otherwise have had, his gains in external rotation and where in space he can now position his arm far outweigh his limitations. For instance, he refused to even try to walk before surgery because if he fell to his left side he would crack his head as his arm could not reach out to protect him. He walked 3 days after his cast came off, and now has great protective reflexes. His arm is unbelievably more functional and "normal" looking since his surgery, and all the things he can do now greatly outweigh the areas where he was weakened. His only limitations in his 3-year-old gymnastics class are things related to handstands (because he cannot fully abduct his arm) and hand-on-hip. He can do everything else, whereas before surgery his arm was stuck crossed front of him and he couldn't even crawl properly.

In addition, hopefully he now has a stable shoulder for future growth which we pray means that he won't sublux/dislocate and lose function later as he grows, as so many kids do. That is conjecture at this point, though.... only time will tell if a normalized shoulder joint will prevent future functional losses as the child grows to adulthood. We were betting that it would when we chose this surgery.

So yes, there is a very real risk of loss of internal rotation. I would only go with a doctor who has been really tracking this risk and has a clear appreciation for what it means. For us, it meant doing only ACR without tendon transfers, to "see what we have" before doing anything else. Now, even if my son could get more overhead with tendon transfers, his surgeon strongly advises against it because it might tip the balance too far into external rotation. I respect his caution when it comes to this surgery. It can be a miracle surgery, but proceed with caution because there ARE risks.


Kate

I have never heard of ACR can someone explain what this is?